Hereditary ataxia

hereditary ataxia: A hereditary disorder consisting of degeneration of the cerebellum and/or of the spine; most cases feature both to some extent, and therefore present with overlapping cerebellar and sensory ataxia, even though one is often more evident than the other. Hereditary disorders causing ataxia include autosomal dominant ones such as spinocerebellar ataxia, episodic ataxia, and dentatorubropallidoluysian atrophy, as well as autosomal recessive disorders such as Friedreich's ataxia (sensory and cerebellar, with the former predominating) and Niemann Pick disease, ataxia-telangiectasia (sensory and cerebellar, with the latter predominating), and abetalipoproteinaemia. An example of X-linked ataxic condition is the rare fragile X-associated tremor/ataxia syndrome or FXTAS.

Endpoint definition

FinnGen phenotype data
diagram bullet diagram downward connector

Filter registries Inpat., Oupat., Death

Hospital Discharge: ICD-10 G11
Hospital discharge: ICD-9 334
Hospital discharge: ICD-8 332
Cause of death: ICD-10 G11
Cause of death: ICD-9 334
Cause of death: ICD-8 332

Check pre-conditions None

Include endpoints None

Check conditions None

Apply sex-specific rule None

diagram downward connector G6_HERATAXIA

Extra metadata

Level in the ICD hierarchy 3
First used in FinnGen datafreeze DF2
Parent code in ICD-10 G10-G13
Name in latin Ataxia hereditaria

Summary Statistics

Key figures

All Female Male
Number of individuals 194 85 109
Unadjusted prevalence (%) 0.07 0.06 0.10
Mean age at first event (years) 50.96 49.05 52.46

Mortality

Follow-up Absolute risk HR [95% CI] p N
1998–2019 - - - -
15 years - - - -
5 years - - - -
1 year - - - -

Correlations

Index endpoint: G6_HERATAXIA – Hereditary ataxia
GWS hits: 1

Survival analyses between endpoints

Plot

before Hereditary ataxia
after Hereditary ataxia

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Drugs most likely to be purchased after Hereditary ataxia